Primary Sclerosing Cholangitis – Causes, Symptoms, Diagnosis, & Treatment
What is primary sclerosing cholangitis?
Primary sclerosing cholangitis (PSC) is a chronic disease that progressively damages the bile ducts. This disease is characterized by inflammation (swelling), scarring, and destruction of the bile ducts inside and outside of the liver.
Primary sclerosing cholangitis occurs more often in men than women. This disease is rare in children.
What causes primary sclerosing cholangitis?
What causes primary sclerosing cholangitis is not known in most cases, it is thought that the body’s immune system attacks bile ducts and cause inflammation. It is also thought that genetic factors may be responsible for this disorder.
PSC is associated with the following conditions:
- Inflammatory bowel disease (ulcerative colitis and Crohn’s disease)
- Autoimmune disorders
- Chronic pancreatitis
- Sarcoidosis (a disease that causes inflammation in various parts of the body)
Primary sclerosing cholangitis may also be caused by the following conditions:
- Choledocholithiasis (gallstones in the bile duct)
- Infections in the liver, gallbladder, and bile ducts
What are the symptoms of primary sclerosing cholangitis?
PMS causes no symptoms initially in some people. People can have the disease without symptoms for many years.
The following are the usual first symptoms in the initial stages of the disease:
- Jaundice (yellowing of the skin and eyes)
The following symptoms may appear as the disease progresses:
- Enlarged liver
- Enlarged spleen
- Loss of appetite
- Weight loss
- Recurring episodes of cholangitis
How is primary sclerosing cholangitis diagnosed?
The doctor will perform a physical exam and review your symptoms. Some people without symptoms may have abnormal liver function test results. In such people, the doctor will try to identify other diseases that cause similar problems, diseases that often occur with PSC, such as inflammatory bowel disease and gallstones.
The doctor may order the following tests to diagnose PSC:
- Abdominal CT scan
- Abdominal ultrasound
- Endoscopic retrograde cholangiopancreatography (ERCP)
- Liver biopsy
- Magnetic resonance cholangiopancreatography (MRCP)
- Percutaneous transhepatic cholangiogram (PTC)
How is primary sclerosing cholangitis treated?
The treatment for PSC is primarily aimed at controlling cholestatic symptoms and preventing complications.
Treatment may include medication to reduce itching and jaundice, antibiotics to treat infections, and vitamin supplements since people with PSC are often deficient in vitamins A, D, E, and K.
In some cases, surgery may be used to open major blockages in the common bile duct.
Liver transplantation may be required in some cases.
This feature is for informational purposes only and is not intended to substitute the expert guidance of a doctor. We advise seeing a doctor if you have any health concerns.